- Pictorial Review
- Open Access
The complex of myxomas, spotty skin pigmentation and endocrine overactivity (Carney complex): imaging findings with clinical and pathological correlation
© The Author(s) 2013
- Received: 15 July 2012
- Accepted: 16 November 2012
- Published: 12 January 2013
The complex of myxomas, spotty skin pigmentation and endocrine overactivity, or Carney complex (CNC), is a familial multiple endocrine neoplasia and lentiginosis syndrome. CNC is inherited in an autosomal dominant manner and is genetically heterogeneous. Its features overlap those of McCune-Albright syndrome and other multiple endocrine neoplasia (MEN) syndromes. Spotty skin pigmentation is the major clinical manifestation of the syndrome, followed by multicentric heart myxomas, which occur at a young age and are the lethal component of the disease. Myxomas may also occur on the skin (eyelid, external ear canal and nipple) and the breast. Breast myxomas, when present, are multiple and bilateral among female CNC patients, an entity which is also described as “breast-myxomatosis” and is a characteristic feature of the syndrome. Affected CNC patients often have tumours of two or more endocrine glands, including primary pigmented nodular adrenocortical disease (PPNAD), an adrenocorticotropin hormone (ACTH)-independent cause of Cushing’s syndrome, growth hormone (GH)-secreting and prolactin (PRL)-secreting pituitary adenomas, thyroid adenomas or carcinomas, testicular neoplasms (large-cell calcifying Sertoli cell tumours [LCCSCT]) and ovarian lesions (cysts and cancinomas). Additional infrequent but characteristic manifestations of CNC are psammomatous melanotic schwannomas (PMS), breast ductal adenomas (DAs) with tubular features, and osteochondromyxomas or “Carney bone tumour”.
• Almost 60 % of the known CNC kindreds have a germline inactivating mutations in the PRKAR1A gene.
• Spotty skin pigmentation is the major clinical manifestation of CNC, followed by heart myxomas.
• Indicative imaging signs of PPNAD are contour abnormality and hypodense spots within the gland.
• Two breast tumours may present in CNC: myxoid fibroadenomas (breast myxomatosis) and ductal adenomas.
• Additional findings of CNC are psammomatous melanotic schwannomas (PMSs) and osteochondromyxomas.
- Carney complex
- Protein kinase A
- Spotty skin pigmentation
- Primary pigmented nodular adrenocortical disease
- Large-cell calcifying sertoli cell tumour
- Psammomatous melanotic schwannoma
- Ductal adenoma
The complex of myxomas, spotty skin pigmentation and endocrine overactivity, or Carney complex (CNC) (MIM No. 160980), is a familial lentiginosis and multiple endocrine neoplasia syndrome described in 1985 by Carney [1, 2]. More than 500 patients with CNC have been reported since then, worldwide, and the majority of them (almost 70 %) presented with positive family history [3, 4]. The median age at detection is 20 years, but the initial presentation varies among patients. Life expectancy of patients with CNC is decreased mainly due to heart-related causes, which account for 57 % of deaths of CNC patients . Noteworthy, CNC is different from Carney triad. Although the two conditions share part of their names, Carney triad presents with gastrointestinal stromal tumours (GISTs), lung chondromas, paragangliomas, adrenal adenomas and pheochromocytomas, oesophageal leiomyomas and other hamartomatous lesions.
Linkage analysis studies have identified two CNC genetic loci: (1) a 6.4-cM region on chromosome 2 (2p16) and (2) a 17-cM region on chromosome 17 (17q22-24) [6, 7]. Almost 60 % of the known CNC kindreds have a germline inactivating mutation in the PRKAR1A gene, which codes for the regulatory subunit type 1α (RIa) of the cAMP-dependent protein kinase A (PKA), located on chromosome 17q23-24 [4, 8]. Inactivating mutations of the PRKAR1A gene lead to aberrant function of PKA and increased phosphorylation of targets implicated in cell transcription, metabolism, cell cycle progression and apoptosis . The PRKAR1A gene seemingly functions as a tumour suppressor gene in tumours of CNC patients [10, 11]. The penetrance for CNC due to PRKAR1A defects is close to 100 % by the age of 50, but this does not apply to kindreds with CNC caused by other genetic defects [5, 12]. Although CNC has a similar presentation as other lentiginosis syndromes, like Peutz-Jeghers syndrome, Cowden disease and Bannayan-Zonana syndrome, studies have failed to demonstrate any correlation of their genetic basis [13–18].
Diagnostic criteria for CNC (from  with written permission)
Diagnostic criteria for CNC
• Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)
• Myxoma (cutaneous and mucosal)a
• Cardiac myxomaa
• Breast myxomatosisa or fat-suppressed MRI findings suggestive of this diagnosis
• PPNADa or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test
• Acromegaly due to GH-producing adenomaa
• LCCSCTa or characteristic calcification on testicular ultrasonography
• Thyroid carcinoma (at any age)a or multiple, hypoechoic nodules on thyroid ultrasonography in a prepubertal child
• Psammomatous melanotic schwannomaa
• Blue nevus, epithelioid blue nevus (multiple)a
• Breast ductal adenoma (multiple)a
1. Affected first-degree relative
2. Inactivating mutation of the PRKAR1A gene
Skin abnormalities are present in almost 77 % of the CNC patients . The most common skin lesions are spotty skin pigmentations (lentigines), epithelioid blue-nevi and skin myxomas. Rare skin manifestations include: ephelides (hyperpigmentation of the basal epidermal unit), junctional nevi (nodular accumulation of melanocytes in the dermis-epidermis junction), café-au-lait spots and atypical blue or compound nevi. Many patients present with a combination of the aforementioned skin lesions.
Spotty skin pigmentations
Lentigines are characterised by hyperpigmentation of the basal epidermal layer with hyperplasia of melanocytes and increased melanin in melanocytes and basal keratinocytes, with or without elongation of the rete ridges.
Histologically, epithelioid blue nevi are characterised by short fascicles, small nests and single, round or spindle-shaped melanocytes with dendritic, pigmented, elongation processes in the upper dermis. Mitotic activity is rare.
Skin myxomas consist of basophilic ground substance (myxoid stroma) with few macrophages or bland spindle cells, forming poorly or well-lobulated dermal masses with widely spaced capillaries (Fig. 3b).
Among CNC patients, heart myxomas occur at any age (median age at detection, 20 years), although at a younger age when compared with sporadic cardiac myxomas. They may be multicentric and can be found in all cardiac chambers [5, 19]. The presenting signs and symptoms derive from cardiac or embolic events, such as embolic strokes. Heart myxomas and emboli deriving from them are the main cause of morbidity and mortality of CNC patients. Frequently, CNC patients with heart myxomas have two or more operations for recurrent tumours [19, 20].
Pituitary gland: growth hormone (GH)-secreting and prolactin (PRL)-secreting adenomas
The incidence of GH-producing pituitary tumours and clinical acromegaly has been estimated at less than 15 % in patients with CNC [10, 21]. However, abnormal response of GH to oral glucose tolerance test and “paradoxical” response to stimuli, such as thyrotropin-releasing hormone (TRH), without a detectable tumour in MRI studies, may be present in up to 80 % of the patients . Acromegaly, when present, is generally characterised by a slow, progressive clinical course. Patients presenting with acromegaly as the primary manifestation of the CNC usually have pituitary macroadenomas (tumour size bigger than 10 mm).
The histology reports from CNC patients operated on for pituitary tumours suggest that the pituitary adenomas are usually surrounded by a hyperplastic transition zone and multiple tumours are concurrently present. This finding, along with data from genetic studies of the adenomas, supports the concept that each adenoma stems from clonic undifferentiation of hyperplastic pituitary cells . The adenomas usually stain positive for GH and PRL, and occasionally for α-subunit, β-TSH and β-LH .
In a study where patients were followed-up prospectively, increased levels of GH and PRL were usually found before the radiological detection of the tumour . In the majority of these cases, the tumours were microadenomas (greatest diameter less than 1 cm) and the radiological assessment of the pituitary gland was negative [21, 23, 24].
Thyroid gland: adenomas, carcinomas
In the biggest published series to date with sonographic evaluation of patients with the complex, ultrasonography revealed some thyroid pathology in almost 60 % of all patients, and 67 % among children and adolescents . This comes in contrast with the detection rate of unsuspected nodules in the general population which varies from 17 to 19 % in men, 20–44 % in women and up to 1.8 % in children [26–28]. Hence, CNC patients, despite the sonographic findings, usually remain clinically and biochemically euthyroid (levels of T4, Free T4, T3, and TSH remain within normal limits) .
Adrenal glands: primary pigmented nodular adrenocortical disease (PPNAD)
PPNAD is a generally rare cause of adrenocorticotropin hormone (ACTH)-independent Cushing’s syndrome, but it is observed in almost 25–30 % of CNC patients. However, histological evidence of PPNAD has been reported in almost every patient with CNC who underwent an autopsy . PPNAD usually presents in the 2nd or 3rd decade of life, without demonstrating the strong female predilection seen in adult patients with Cushing’s syndrome . The disease is named after the small, cortisol producing, pigmented micronodules seen in the adrenal cortex . The hypercortisolism may manifest as the classic constellation of Cushing’s characteristics or with isolated features, like mild growth retardation (short stature), severe precocious osteoporosis and severe muscle and skin wasting . Cyclical or atypical Cushing’s syndrome is also common among patients with CNC . Biochemically, the blood and urinary cortisol levels are elevated, while ACTH levels are low if not undetectable. In Liddle’s test (using low and high doses of dexamethasone), these patients usually present with a paradoxical increase of urinary free cortisol and 17-hydroxycorticosteroid levels on the 2nd day of administration of high doses of dexamethasone . The recommended treatment for patients with PPNAD is bilateral adrenalectomy [33, 34].
Two different breast tumours, which can coexist, have been reported in CNC patients: (1) myxoid fibroadenomas (“breast myxomatosis”), which are abnormalities of the mesenchyma, and (2) ductal adenomas (DAs), which are abnormalities of the epithelium [36, 37]. Myxoid fibroadenomas and DAs are benign and no malignant transformation has been reported among CNC patients [36, 38]. However, the possibility that the benign hyperplastic lesions progress to malignant ones cannot be excluded. Additionally, the presence of these lesions usually complicates the mammographic screening of patients with CNC for breast cancer, because of their multiplicity and the similar imaging characteristics.
Myxoid fibroadenomas or “breast myxomatosis”
The physical examination of the breast with myxoid fibroadenomas is significant for diffuse nodularity without dominant masses, probably due to the young age of the examined patients (3rd or 4th decade of life). None of the known patients with isolated myxoid fibroadenomas has been reported to have blood nipple discharge, breast skin abnormalities or sentinel lymphadenopathy . The lesions present bilaterally and multifocally in almost 40 % of the patients .
Ductal adenomas (DAs)
The ductal adenomas of the breast were first reported by Azzopardi and Salm in 1984 . It should be noticed that in that series, due to clinical (presentation in older patients), imaging (presence of suspicious microcalcifications), and pathological findings (cellularity and complexity pattern with invasion of the tumour capsule), these tumours were misinterpreted as breast carcinomas and two of those patients underwent radical mastectomy . Therefore, it is important to recognise DAs for two reasons. First, to avoid their misinterpretation as breast carcinoma and second, because they are a characteristic pathological entity for CNC as described by Carney and Toorkey . The presentation of DA could be even the primary manifestation of the complex.
Usually, DAs are palpable, painless masses near the areola, which can be asymptomatic or manifest as blood nipple discharge.
Primarily large-cell calcifying sertoli cell tumour (LCCSCT)
The most frequent testicular manifestation in the CNC is the LCCSCT, although Leydig cell tumours and nodular adrenocortical rest tissue have also been reported . LCCSCTs are almost always found by ultrasonography in post-pubertal male patients with CNC, although the age at diagnosis ranges from 2 to 51 years old [10, 40]. LCCSCTs have also been reported in patients with Peutz-Jeghers syndrome, underlining furthermore the similarity of these two syndromes . The initial treatment applied to these patients was tumour enucleation or partial orchiectomy . However, the current recommendations suggest annual sonographic follow-up and measurement of testicular tumour markers for patients with CNC and LCCSCT, because of the potential risk for malignant transformation [5, 10].
LCCSCTs, often bilateral and multicentric, are usually asymptomatic, but can rarely present with precocious puberty or gynaecomastia. Men with CNC are also at increased risk for infertility [40, 41]. In clinical examination, tumours, when palpable, are often felt as hard non-tender masses.
Myxomas, ovarian cysts, ovarian carcinomas
Carcinomas of the ovarian surface epithelium have been identified in 1.12 % of female patients with CNC, whereas the reported incidence of ovarian cancer among American women is 0.015 % [42, 43]. In an autopsy series of 12 female patients affected by the complex, 58 % were found to have an ovarian lesion, while 67 % of women with CNC participating in a prospective sonographic study had at least 1 ultrasound study positive for ovarian cysts . Non-stromal tumours (like in Peutz-Jeghers syndrome) have also been reported among patients with the complex .
The sonographic findings have no characteristic feature and are similar to the non-specific multilocular ovarian lesions usually seen in the general population. Ultrasound of the ovaries may be part of the initial evaluation in female patients with CNC; follow-up of any identified lesion is recommended, because of the possible risk for malignancy .
Psammomatous melanotic schwannoma (PMS)
PMSs have no characteristic clinical sign, symptom, or imaging finding. They occur commonly in the gastrointestinal tract (favouring the stomach), in the spinal nerve roots and can rarely involve the trigeminal nerve, also eroding the bones . Another not uncommon site is the chest wall with involvement of adjacent ribs. PMSs, as classic schwannomas, can also involve the spinal nerves, when they are located in their posterior root . PMSs are potentially malignant and cases of patients dying from metastasis have been described .
Osteochondromyxoma or “Carney bone tumour”
Although congenital bone tumours are very rare, an unusual type, osteochondromyxomas, may present within the context of CNC [47, 48]. Only a few cases have been described until now and none of them has metastasised, even though some cases presented with osteolytic appearance .
In all reported patients, osteochondromyxomas were painless. Tumours were detected during the imaging evaluation of mass side effects, like proptosis, nasal obstruction, or painless swelling .
The CNC may present with symptoms from a variety of different tissues and can mimic isolated diseases or other syndromes. The recognition of the strong association of certain manifestations with the complex and the specific constellation of symptoms that these patients experience is important for establishing the appropriate diagnosis and treatment.
Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.
- Carney JA (1985) The complex of myxomas, spotty skin pigmentation and endocrine overactivity. Medicine 64(4):270–283View ArticlePubMedGoogle Scholar
- OMIM (12/10/2010) MIM number: #160980. Johns Hopkins University, Baltimore, MD. World Wide Web URL: http://omimorg/
- Rothenbuhler A, Stratakis CA (2010) Clinical and molecular genetics of Carney complex. Best Pract Res Clin Endocrinol Metab 24(3):389–399View ArticlePubMedGoogle Scholar
- Bertherat J, Horvath A, Groussin L, Grabar S, Boikos S, Cazabat L, Libe R, Rene-Corail F, Stergiopoulos S, Bourdeau I, Bei T, Clauser E, Calender A, Kirschner LS, Bertagna X, Carney JA, Stratakis CA (2009) Mutations in regulatory subunit type 1A of cyclic adenosine 5′-monophosphate-dependent protein kinase (PRKAR1A): phenotype analysis in 353 patients and 80 different genotypes. J Clin Endocrinol Metab 94(6):2085–2091PubMed CentralView ArticlePubMedGoogle Scholar
- Stratakis CA, Kirschner LS, Carney JA (2001) Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86(9):4041–4046View ArticlePubMedGoogle Scholar
- Casey M, Mah C, Merliss AD, Kirschner LS, Taymans SE, Denio AE, Korf B, Irvine AD, Hughes A, Carney JA, Stratakis CA, Basson CT (1998) Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation 98(23):2560–2566View ArticlePubMedGoogle Scholar
- Stratakis CA, Carney JA, Lin JP, Papanicolaou DA, Karl M, Kastner DL, Pras E, Chrousos GP (1996) Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest 97(3):699–705PubMed CentralView ArticlePubMedGoogle Scholar
- Kirschner LS, Carney JA, Pack SD, Taymans SE, Giatzakis C, Cho YS, Cho-Chung YS, Stratakis CA (2000) Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex. Nat Genet 26(1):89–92View ArticlePubMedGoogle Scholar
- Bossis I, Stratakis CA (2004) Minireview: PRKAR1A: normal and abnormal functions. Endocrinology 145(12):5452–5458View ArticlePubMedGoogle Scholar
- Boikos SA, Stratakis CA (2007) Carney complex: the first 20 years. Curr Opin Oncol 19(1):24–29View ArticlePubMedGoogle Scholar
- Boikos SA, Stratakis CA (2006) Carney complex: pathology and molecular genetics. Neuroendocrinology 83(3–4):189–199View ArticlePubMedGoogle Scholar
- Horvath A, Bertherat J, Groussin L, Guillaud-Bataille M, Tsang K, Cazabat L, Libe R, Remmers E, Rene-Corail F, Faucz FR, Clauser E, Calender A, Bertagna X, Carney JA, Stratakis CA (2010) Mutations and polymorphisms in the gene encoding regulatory subunit type 1-alpha of protein kinase A (PRKAR1A): an update. Hum Mutat 31(4):369–379PubMed CentralView ArticlePubMedGoogle Scholar
- Chrousos GP, Stratakis CA (1998) Carney complex and the familial lentiginosis syndromes: link to inherited neoplasias and developmental disorders, and genetic loci. J Intern Med 243(6):573–579View ArticlePubMedGoogle Scholar
- Stratakis CA (2000) Genetics of Carney complex and related familial lentiginoses, and other multiple tumor syndromes. Front Biosci 5:D353–D366View ArticlePubMedGoogle Scholar
- Stratakis CA, Kirschner LS, Taymans SE, Tomlinson IP, Marsh DJ, Torpy DJ, Giatzakis C, Eccles DM, Theaker J, Houlston RS, Blouin JL, Antonarakis SE, Basson CT, Eng C, Carney JA (1998) Carney complex, Peutz-Jeghers syndrome, Cowden disease, and Bannayan-Zonana syndrome share cutaneous and endocrine manifestations, but not genetic loci. J Clin Endocrinol Metab 83(8):2972–2976View ArticlePubMedGoogle Scholar
- Stratakis CA (2001) Clinical genetics of multiple endocrine neoplasias, Carney complex and related syndromes. J Endocrinol Investig 24(5):370–383View ArticleGoogle Scholar
- Stratakis CA (2000) Genetics of Peutz-Jeghers syndrome, Carney complex and other familial lentiginoses. Horm Res 54(5–6):334–343View ArticlePubMedGoogle Scholar
- Stratakis C (1999) Carney complex and related syndromes and their genetic loci—author’s response. J Clin Endocrinol Metab 84(4):1491–1492View ArticleGoogle Scholar
- McCarthy PM, Piehler JM, Schaff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr, Carney JA (1986) The significance of multiple, recurrent, and “complex” cardiac myxomas. J Thorac Cardiovasc Surg 91(3):389–396PubMedGoogle Scholar
- Carney JA (1995) Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol 14(2):90–98View ArticlePubMedGoogle Scholar
- Pack SD, Kirschner LS, Pak E, Zhuang Z, Carney JA, Stratakis CA (2000) Genetic and histologic studies of somatomammotropic pituitary tumors in patients with the “complex of spotty skin pigmentation, myxomas, endocrine overactivity and schwannomas” (Carney complex). J Clin Endocrinol Metab 85(10):3860–3865PubMedGoogle Scholar
- Boikos SA, Stratakis CA (2006) Pituitary pathology in patients with Carney Complex: growth-hormone producing hyperplasia or tumors and their association with other abnormalities. Pituitary 9(3):203–209View ArticlePubMedGoogle Scholar
- Watson JC, Stratakis CA, Bryant-Greenwood PK, Koch CA, Kirschner LS, Nguyen T, Carney JA, Oldfield EH (2000) Neurosurgical implications of Carney complex. J Neurosurg 92(3):413–418View ArticlePubMedGoogle Scholar
- Raff SB, Carney JA, Krugman D, Doppman JL, Stratakis CA (2000) Prolactin secretion abnormalities in patients with the “syndrome of spotty skin pigmentation, myxomas, endocrine overactivity and schwannomas” (Carney complex). J Pediatr Endocrinol Metab 13(4):373–379PubMedGoogle Scholar
- Stratakis CA, Courcoutsakis NA, Abati A, Filie A, Doppman JL, Carney JA, Shawker T (1997) Thyroid gland abnormalities in patients with the syndrome of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas (Carney complex). J Clin Endocrinol Metab 82(7):2037–2043View ArticlePubMedGoogle Scholar
- Ezzat S, Sarti DA, Cain DR, Braunstein GD (1994) Thyroid incidentalomas. Prevalence by palpation and ultrasonography. Arch Intern Med 154(16):1838–1840View ArticlePubMedGoogle Scholar
- Mortensen J, Woolner LB, Bennett WA (1955) Gross and microscopic findings in clinically normal thyroid glands. J Clin Endocrinol Metab 15(10):1270–1280View ArticlePubMedGoogle Scholar
- Garcia CJ, Daneman A, Thorner P, Daneman D (1992) Sonography of multinodular thyroid gland in children and adolescents. Am J Dis Child 146(7):811–816PubMedGoogle Scholar
- Cetta F (1997) Familial nonmedullary thyroid carcinomas: a heterogeneous syndrome with different natural history and variable long-term prognosis. J Clin Endocrinol Metab 82(12):4274–4275View ArticlePubMedGoogle Scholar
- Stratakis CA, Kirschner LS (1998) Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease) leading to Cushing syndrome. Horm Metab Res 30(6–7):456–463View ArticlePubMedGoogle Scholar
- Shenoy BV, Carpenter PC, Carney JA (1984) Bilateral primary pigmented nodular adrenocortical disease. Rare cause of the Cushing syndrome. Am J Surg Pathol 8(5):335–344View ArticlePubMedGoogle Scholar
- Stratakis CA, Sarlis N, Kirschner LS, Carney JA, Doppman JL, Nieman LK, Chrousos GP, Papanicolaou DA (1999) Paradoxical response to dexamethasone in the diagnosis of primary pigmented nodular adrenocortical disease. Ann Intern Med 131(8):585–591View ArticlePubMedGoogle Scholar
- Grant CS, Carney JA, Carpenter PC, van Heerden JA (1986) Primary pigmented nodular adrenocortical disease: diagnosis and management. Surgery 100(6):1178–1184PubMedGoogle Scholar
- Sarkar R, Thompson NW, McLeod MK (1990) The role of adrenalectomy in Cushing’s syndrome. Surgery 108(6):1079–1084PubMedGoogle Scholar
- Doppman JL, Travis WD, Nieman L, Miller DL, Chrousos GP, Gomez MT, Cutler GB Jr, Loriaux DL, Norton JA (1989) Cushing syndrome due to primary pigmented nodular adrenocortical disease: findings at CT and MR imaging. Radiology 172(2):415–420View ArticlePubMedGoogle Scholar
- Carney JA, Toorkey BC (1991) Myxoid fibroadenoma and allied conditions (myxomatosis) of the breast. A heritable disorder with special associations including cardiac and cutaneous myxomas. Am J Surg Pathol 15(8):713–721View ArticlePubMedGoogle Scholar
- Carney JA, Toorkey BC (1991) Ductal adenoma of the breast with tubular features. A probable component of the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Am J Surg Pathol 15(8):722–731View ArticlePubMedGoogle Scholar
- Courcoutsakis NA, Chow CK, Shawker TH, Carney JA, Stratakis CA (1997) Syndrome of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas (Carney complex): breast imaging findings. Radiology 205(1):221–227View ArticlePubMedGoogle Scholar
- Azzopardi JG, Salm R (1984) Ductal adenoma of the breast: a lesion which can mimic carcinoma. J Pathol 144(1):15–23View ArticlePubMedGoogle Scholar
- Washecka R, Dresner MI, Honda SA (2002) Testicular tumors in Carney’s complex. J Urol 167(3):1299–1302View ArticlePubMedGoogle Scholar
- Wieacker P, Stratakis CA, Horvath A, Klose S, Nickel I, Buhtz P, Muschke P (2007) Male infertility as a component of Carney complex. Andrologia 39(5):196–197View ArticlePubMedGoogle Scholar
- Stratakis CA, Papageorgiou T, Premkumar A, Pack S, Kirschner LS, Taymans SE, Zhuang Z, Oelkers WH, Carney JA (2000) Ovarian lesions in Carney complex: clinical genetics and possible predisposition to malignancy. J Clin Endocrinol Metab 85(11):4359–4366View ArticlePubMedGoogle Scholar
- Crayford TJ, Campbell S, Bourne TH, Rawson HJ, Collins WP (2000) Benign ovarian cysts and ovarian cancer: a cohort study with implications for screening. Lancet 355(9209):1060–1063View ArticlePubMedGoogle Scholar
- Papageorgiou T, Stratakis CA (2002) Ovarian tumors associated with multiple endocrine neoplasias and related syndromes (Carney complex, Peutz-Jeghers syndrome, von Hippel-Lindau disease, Cowden’s disease). Int J Gynecol Cancer 12(4):337–347View ArticlePubMedGoogle Scholar
- Carney JA (1990) Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 14(3):206–222View ArticlePubMedGoogle Scholar
- Utiger CA, Headington JT (1993) Psammomatous melanotic schwannoma. A new cutaneous marker for Carney’s complex. Arch Dermatol 129(2):202–204View ArticlePubMedGoogle Scholar
- Siegal GP (2001) Osteochondromyxoma of bone: a lot more than fodder for the spelling-challenged. Am J Surg Pathol 25(2):268View ArticlePubMedGoogle Scholar
- Carney JA, Boccon-Gibod L, Jarka DE, Tanaka Y, Swee RG, Unni KK, Stratakis CA (2001) Osteochondromyxoma of bone: a congenital tumor associated with lentigines and other unusual disorders. Am J Surg Pathol 25(2):164–176View ArticlePubMedGoogle Scholar